bims-tremyl Biomed News
on Therapy resistance biology in myeloid leukemia
Issue of 2026–03–08
24 papers selected by
Paolo Gallipoli, Barts Cancer Institute, Queen Mary University of London



  1. Blood Adv. 2026 Mar 05. pii: bloodadvances.2025018188. [Epub ahead of print]
      Secondary acute myeloid leukemia (AML) comprises heterogeneous entities, unified by poor prognosis, defined by patients' previous history. We evaluated the associations of genetic profiles with blast counts and patients' history in a cohort of 924 patients with MDS/AML or AML, classified according to the International Consensus Classification (ICC). The cohort included 109 cases with "mutated TP53", 497 with "myelodysplasia-related (MDR) gene mutation" and 93 with "MDR-cytogenetic abnormality", 77 therapy-related and 136 "not otherwise specified" (NOS) AML as controls. Exploring the ICC hierarchy, "mutated TP53" and "MDR-cytogenetic abnormality" AML and MDS/AML categories presented similar biology and prognosis, irrespective of blast counts. Conversely, in MDS/AML with "MDR gene mutation" and NOS, profiles significantly differed from AML and characterized by a higher number of mutations in STAG2, SRSF2, ASXL1 and TET2. This corresponded to improved survival in MDS/AML vs AML (MDR-gene mutation: median OS 24.8 vs. 13.6 months, p<0.0001, and NOS: 49.9 vs. 19.2 months, p=0.028). Within each ICC-defined AML category, a prior MDS history versus de novo onset did not impact on patients' prognosis. We then analyzed secondary AML, defined by "prior MDS or MDS/MPN" or "therapy-related" (t-AML), as diagnostic qualifiers. According to ELN 2022, AML post-MDS mostly clustered in the adverse-risk group (84.1%), while t-AML showed more heterogeneous ELN profiles (12.9% favorable, 33.8% intermediate, and 53.3% adverse risk) reflecting diverse overall survival. Our findings underscore that genetic features and the ICC classification reliably capture disease biology, refine risk stratification, and ultimately guide treatment decisions in most secondary AML and MDS/AML.
    DOI:  https://doi.org/10.1182/bloodadvances.2025018188
  2. Blood. 2026 Mar 05. pii: blood.2025031858. [Epub ahead of print]
      Briquilimab is a monoclonal antibody inhibiting stem cell factor (SCF) binding to CD117 (c-Kit). Based on preclinical data demonstrating the antibody clears hematopoietic stem and progenitor cells (HSPC) and myeloid malignant cells, we conducted a phase 1 trial examining briquilimab plus non-myeloablative fludarabine (flu) and total body irradiation (TBI) as conditioning for older adults with acute myeloid leukemia (AML) or myelodysplastic syndrome (MDS) undergoing matched donor allogeneic hematopoietic cell transplantation (HCT). Briquilimab was infused 10-14 days before transplant day (TD) 0; fludarabine 30mg/m2 and TBI 2-3 Gy were administered on TD -4 to -2 and TD0, respectively. Graft-versus-host disease prophylaxis consisted of tacrolimus, sirolimus, and mycophenolate mofetil. Thirty-two patients enrolled (n=13 AML in complete remission [CR], n=3 AML in relapse, n=16 MDS). Median age was 70 years and most had detectable measurable residual disease at screening. There were no briquilimab infusion reactions, dose limiting toxicities, or primary graft failure events; briquilimab clearance was predictable across patients. Among the AML in CR cohort, 1-year EFS was 69.2% (95% CI, 37.3, 87.2); 1-year OS was 75% (95% CI, 40.8, 91.2). Among the MDS cohort, 1-year EFS was 53.8% (26.8, 74.8); 1-year OS was 76.4% (42.7, 91.8). One of 3 AML patients in relapse experienced a transient response. Marrow samples obtained before and after briquilimab and prior to flu/TBI demonstrated AML/MDS HSPC depletion (mean 62.4±22.7%) with resultant 3-fold increase in serum SCF. In summary, we demonstrate the feasibility, safety, and proof of concept of CD117 targeting with briquilimab as HCT conditioning for AML/MDS. The trial is registered at clinicaltrials.gov; using identifier: NCT04429191.
    DOI:  https://doi.org/10.1182/blood.2025031858
  3. Haematologica. 2026 Mar 05.
      FLT3-mutation occurs in 25-30% of AML and confers high relapse risk and inferior survival. Allogeneic hematopoietic cell transplantation (allo-HCT) offers curative potential, yet relapse remains a major post-transplant challenge. Maintenance therapy with FLT3 inhibitors (FLT3i) after allo-HCT has emerged as a promising strategy, but real-world evidence remains limited. This study aimed to assess the impact of FLT3i maintenance on transplant outcomes. We analyzed 523 adults with FLT3-ITD AML in first complete remission who underwent allo-HCT between 2011 and 2023 in 13 German transplant centers participating in the national DRST registry; 22% received FLT3i maintenance (sorafenib 49%, midostaurin 37%, gilteritinib 5%, unknown 9%). In multivariable analyses (MVA), FLT3i maintenance improved OS (HR 2.25, 95% CI [1.28; 3.95], p=0.005), RFS (HR 1.72, 95% CI [1.05; 2.81], p=0.030), non-relapse mortality (HR 3.62, 95% CI [1.08; 12.11], p=0.037), and GVHD- and relapse-free survival (HR 1.59, 95% CI [1.06; 2.40], p=0.025). The cumulative incidence of relapse did not differ. In univariate analyses (UVA), OS benefits were observed in MRD-positive (HR 2.35, 95% CI [1.04; 5.31], p=0.025) and MRD-negative patients (HR 2.64, 95% CI [1.05; 6.68], p=0.020. Sorafenib maintenance (n=50) demonstrated superior efficacy with 5-year OS of 85% versus 62% (HR 2.979, p=0.0045) and RFS of 84% versus 55% (HR 2.771, p=0.0043) compared to no maintenance. These real-world findings, while limited by the retrospective design and potential selection bias, align with randomized trial data and support the use of FLT3i maintenance as part of post-transplant care for FLT3-ITD AML.
    DOI:  https://doi.org/10.3324/haematol.2025.300176
  4. Blood. 2026 Mar 05. pii: blood.2025031366. [Epub ahead of print]
      Secondary myelofibrosis (SMF) represents a late stage of polycythemia vera and essential thrombocythemia, with overall survival (OS) currently defined by the MYelofibrosis SECondary to PV and ET (MYSEC) Prognostic Model (MYSEC-PM). To identify additional myeloid neoplasm-associated cancer gene variants (CGVs) associated with SMF outcome, we evaluated next-generation sequencing panel testing in 644 patients within the MYSEC cohort. Overall, 429 (66.6%) subjects reported at least one CGV, with ASXL1, TET2 and DNMT3A being the most frequently involved. Specific molecular profiles affected OS (p < .001): U2AF1, TP53 or SRSF2 variants (UTS, 9.3% of cases, median OS 4.1 years) and ASXL1 without UTS (25.3%, median OS 8.4 years). By integrating these genetic signatures within the MYSEC-PM through penalized Cox regressions, we identified the following independent predictors (p from < .0001 to .02) and weighted: hemoglobin <11 g/dl (1 point), circulating blasts ⩾3% (2), platelets <150 × 109/l (2), age (0.21 points/year), ASXL1 without UTS mutations (1) and any UTS mutations (3). Finally, we developed the MYSEC-molecular prognostic model (MYSEC-mPM) allocating 582 SMF patients into four categories with different OS (p < .001): low (median OS 18.0 years, 95%CI: 14.2-not reached; score <14), intermediate-1 (8.8. years, 95%CI: 7.7-9.7; score 14-16), intermediate-2 (4.6 years, 95%CI: 3.1-7.2; score 17-18), and high risk (1.9 years, 95%CI: 1.2-2.5; score ⩾19). Additionally, in 381 SMF with available cytogenetics, the MYSEC-mPM was implemented with complex/monosomal karyotype, generating the karyotype-enhanced MYSEC-kmPM. Our study shows that genomic and cytogenetic profiling improve survival prediction in SMF, outperforming the MYSEC-PM.
    DOI:  https://doi.org/10.1182/blood.2025031366
  5. Lancet Haematol. 2026 Mar;pii: S2352-3026(25)00358-8. [Epub ahead of print]13(3): e157-e168
       BACKGROUND: Patients with relapsed or refractory acute myeloid leukaemia have a poor prognosis. Outcomes with conventional intensive salvage chemotherapy are suboptimal. We aimed to evaluate the safety and activity of high-dose cytarabine and mitoxantrone in combination with venetoclax in patients with relapsed or refractory acute myeloid leukaemia.
    METHODS: This study was a multicentre, open-label, single-arm, phase 1/2 trial. Patients aged 18-75 years, medically fit for intensive chemotherapy (ie, Eastern Cooperative Oncology Group performance status ≤2, adequate hepatic and renal function) with relapsed (haematological relapse from first or second complete remission) or refractory acute myeloid leukaemia (no response after one or two courses of standard induction chemotherapy) were eligible. Patients received venetoclax 400 mg orally once a day on days 1-14 (initial 3-day dose ramp-up), mitoxantrone 10 mg/m2 intravenously once a day on days 5-7, and cytarabine (dose level 1, 200 mg/m2 continuous intravenous infusion on days 4-10; dose level 2, cytarabine 500 mg/m2 intravenously twice a day on days 3-5; or dose level 3, cytarabine 1000 mg/m2 intravenously twice a day on days 3-5). Phase 1 applied a 3 + 3 dose-escalation design to determine the primary endpoint of maximum tolerated dose and recommended phase 2 dose. The primary endpoint of phase 2 was the composite complete remission rate by intention-to-treat. Safety was assessed in all patients exposed to venetoclax. The trial is registered with EUDRA-CT, 2018-003025-28, and ClinicalTrials.gov, NCT04330820, and is completed.
    FINDINGS: From April 6, 2020, to Aug 31, 2023, 55 patients were enrolled (12 in phase 1 and 43 in phase 2). Median follow-up was 30·8 months (IQR, 26·6-34·1). Median age was 57 years (IQR, 49·0-66·5). 30 (55%) of 55 patients were male and 25 (45%) were female. 25 (45%) of 55 patients had adverse-risk acute myeloid leukaemia according to the European LeukemiaNet 2022 classification and 19 (35%) had previous allogeneic haematopoietic cell transplantation (HCT). The maximum tolerated dose was not reached, and dose level 3 was considered safe and defined as the recommended phase 2 dose. The composite complete remission rate was 75% (95% CI 61-85, 41 of 55 patients). The most common all-grade adverse events were febrile neutropenia (29 [53%] of 55 patients), pneumonia (12 [22%]), and sepsis (12 [22%]). 15 serious adverse events occurred in 14 (13%) patients, of which sepsis and pneumonia were the most common. Potential treatment-related deaths were reported in four patients (sepsis n=3, pneumonia n=1).
    INTERPRETATION: High-dose cytarabine and mitoxantrone plus venetoclax appeared to be safe, showed promising activity, and could be a new therapeutical approach for medically fit patients with relapsed or refractory acute myeloid leukaemia, especially as a bridge to allogeneic HCT.
    FUNDING: AbbVie.
    DOI:  https://doi.org/10.1016/S2352-3026(25)00358-8
  6. Blood Cancer J. 2026 Mar 06.
      Cytogenetic and genomic profiling of acute myeloid leukemia (AML) guides personalized treatment according to ELN2022 recommendations. However, marked outcome variability persists among cytogenetically normal (CN-) patients, representing an unmet clinical need. We used long-read whole-genome sequencing to interrogate the prognostic significance of structural variations (SVs) in a prospective cohort of 162 intensively treated CN-AML patients. After stringent filtering, we identified 5 somatic SVs associated with shorter overall survival (OS) (HR:4.18, p < 0.001) and event-free survival (EFS) (HR:3.59, p < 0.001) in 13% of the patients. Results were validated in a real-world cohort of 149 CN-AML, using target assays. These high-risk SVs (HRVs) operationally defined a "very high-risk" category in the framework of ELN2022, overall resulting in more accurate OS prediction. HRVs were independent of most frequent mutations, particularly FLT3ITD and NPM1mut. Among the latter patients, HRVs independently predicted shorter OS (8.2 months versus not-reached; p < 0.001), EFS (3.5 versus 25.7 months; p < 0.001), and lower complete response rates (66.7% versus 90.1%; p < 0.005). Finally, we provided evidence of transcriptional deregulation of SV-related genes in primary samples and engineered cell models. Current findings support the value of SVs for refining risk stratification in CN-AML, by identifying patients at exceedingly dismal outcome who might benefit from personalized approaches.
    DOI:  https://doi.org/10.1038/s41408-026-01465-3
  7. Blood. 2026 Mar 04. pii: blood.2025031016. [Epub ahead of print]
      Myeloid/Lymphoid neoplasms with FGFR1 rearrangement (M/LN-FGFR1) are rare, heterogenous diseases due to fusion transcripts originated by translocations of FGFR1 with different partners, resulting in constitutive FGFR1-mediated signaling. Presentation varies from chronic myeloid neoplasms to acute leukemia or lymphoma and extramedullary localizations are common. Outside allogeneic stem cell transplantation (ASCT), survival with conventional therapy is dismal, representing an unmet clinical need. We summarize here the data that led to approval of pemigatinib, a FGFR1 inhibitor, showing unprecedented efficacy in M/LN-FGFR1.
    DOI:  https://doi.org/10.1182/blood.2025031016
  8. Blood Adv. 2026 Mar 04. pii: bloodadvances.2025019152. [Epub ahead of print]
      Myeloproliferative neoplasms (MPNs), including essential thrombocythemia (ET), polycythemia vera (PV), and myelofibrosis (MF), are a diverse group of hematopoietic stem cell neoplasms associated with an increased risk of hematologic progression to secondary MF or leukemia and cardiovascular disease (CVD). Elevated total cholesterol levels are traditional CVD risk factors. Patients with MPNs may have reduced cholesterol levels, potentially reflecting an underlying hypermetabolic state. The impact of serum total cholesterol on clinical outcomes in patients with MPNs has not yet been evaluated. We performed a multicenter retrospective cohort study of patients with MPNs who had undergone transthoracic echocardiography (TTE) with available serum lipid levels at time of TTE not on statin therapy. Patients were categorized by total cholesterol level (≥ 150, 120 - 149, and < 120 mg/dL). Outcomes evaluated were MPN disease progression to secondary MF or acute leukemia, major adverse cardiovascular events (MACE), and death. Multivariable competing-risk regression modeling was performed. A total of 305 patients were included of whom 54.4% had total cholesterol ≥ 150, 21.3% 120 - 149, and 24.3% < 120 mg/dL. After a median follow-up of 50.8 months, total cholesterol < 120 was associated with increased risk of MPN disease progression (aSHR 3.00, 95% CI 1.36 - 6.63), heart failure hospitalization (aSHR 3.76, 95% CI 1.77 - 7.96), and all-cause death (aHR 1.87, 95% CI 1.06 - 3.30) vs ≥ 150 mg/dL. Among patients with MPN, low total cholesterol levels were associated with increased risk of MPN disease progression.
    DOI:  https://doi.org/10.1182/bloodadvances.2025019152
  9. Haematologica. 2026 Mar 05.
      Primary resistance to hypomethylating agents (HMAs) remains a major obstacle in the treatment of elderly patients with myelodysplastic syndromes (MDS) and acute myeloid leukemia (AML). An altered integrity of the vascular wall is suspected to contribute to this resistance, yet the underlying molecular mechanisms remain unclear. Here, we show that small extracellular vesicles (sEVs) derived from leukemic cells resistant to decitabine (DAC-R), a commonly used HMA, promote vascular permeability by downregulating tight junction proteins, including ZO-1, occludin, and claudin5, in endothelial cell monolayers. This disruption of vascular integrity may facilitate vascular leakage and leukemic cell dissemination. Mechanistically, DAC-R cells exhibit increased expression of the fucosyltransferase FUT4, driven by the transcription factor TWIST1, leading to enhanced biosynthesis of non-sialylated Lewis X (LeX) structures. FUT4-mediated LeX modification stabilizes intercellular adhesion molecule 3 (ICAM3) on sEVs, and the delivery of LeX-modified ICAM3 to endothelial cells suppresses NF-κB signaling, impairing endothelial barrier function. Functionally, vascular remodeling driven by fucosylated sEVs promotes leukemic dissemination, suggesting that disruption of vascular homeostasis represents an additional layer of therapeutic resistance. These findings define a TWIST1-FUT4-LeX-ICAM3 axis and highlight glycosylation as a critical mediator of vascular microenvironment remodeling in MDS/AML.
    DOI:  https://doi.org/10.3324/haematol.2025.288146
  10. Cell Death Discov. 2026 Mar 03.
      Myeloid leukaemias harboring complex karyotypes present several unrelated cytogenetic abnormalities and form a distinct subset of AML linked to a dismal prognosis. Currently, no effective options are available for the treatment of those patients, and the discovery of novel therapeutic strategies represent an urgent clinical priority. We previously developed a bioinformatic framework for the identification of novel molecular vulnerabilities for disease stratification and treatment and observed SPINK2, a serine protease inhibitor Kazal-type 2, as a novel and promising candidate target in AML, with particularly pronounced effects in complex karyotype patients. Using publicly available bulk and single cell RNA-seq datasets, we discovered a robust association between SPINK2 and cell cycle regulators, most notably S-phase genes. By performing shRNA-mediated genetic manipulation of SPINK2 expression in a complex karyotype AML cell lines, we observed a profound impairment of proliferation coupled with an induction of terminal myeloid commitment. Moreover, SPINK2-deficient FUJIOKA cells revealed a significant association between SPINK2 and MECOM expression, consistent with findings in patients harbouring complex karyotypes, yet absent in other AML subsets from the TARGET-AML cohort. Our findings suggest a novel potential correlation between SPINK2 and MECOM expression in complex karyotype leukemias and warrant further investigation into the underlying molecular mechanisms through which the SPINK2-MECOM axis enforces aberrant self-renewal and the development of novel targeted approaches aimed at modulating its expression in complex karyotypic AML.
    DOI:  https://doi.org/10.1038/s41420-026-02988-1
  11. Blood. 2026 Mar 02. pii: blood.2025031593. [Epub ahead of print]
      VEXAS syndrome is a severe adult-onset autoinflammatory disease caused by somatic mutations in the UBA1 gene, disrupting cytoplasmic ubiquitin-activating enzyme E1 function in hematopoietic progenitors. Its pathogenesis remains poorly understood, particularly the mechanisms by which UBA1 mutations disrupt myeloid cell function in the context of inflammatory stimuli. Here, we combine a genetically engineered THP-1 monocytic model with ex vivo analyses of blood and tissue samples from VEXAS patients to investigate the consequences of the canonical UBA1M41V mutation. We show that UBA1-mutated monocytes exhibit TNF-α-induced cell death, characterized by RIPK1 phosphorylation, and MLKL- and caspase-8-mediated cell death. Importantly, we extend these findings to patient-derived CD14⁺ sorted cells, confirming that these cells undergo aberrant apoptotic and necroptotic cell death. Mechanistically, activation of these cell death pathways appears to be promoted by defective NF-κB-dependent transcriptional responses and reduced cFLIP(L) expression following TNF-α stimulation. UBA1-mutated monocytes also display blunted cytokine responses to Toll-like receptor (TLR) agonists despite preserved TLR expression, linked to an impaired NF-κB response. UBA1M41V-derived macrophages exhibit a pro-inflammatory transcriptional profile with increased chemokine secretion that promotes monocyte recruitment. In addition, these UBA1-mutated macrophages display impaired efferocytosis due to lysosomal dysfunction. Together, these findings reveal a pathogenic axis in VEXAS syndrome linking UBA1 loss of function and defective ubiquitination to RIPK1-mediated inflammatory cell death, impaired antimicrobial signaling, and defective resolution mechanisms. Our study provides novel mechanistic insights into the myeloid dysfunction underlying inflammation and cytopenia in VEXAS and supports the therapeutic targeting of inflammatory cell death pathways.
    DOI:  https://doi.org/10.1182/blood.2025031593
  12. Cell Rep. 2026 Mar 03. pii: S2211-1247(26)00120-8. [Epub ahead of print]45(3): 117042
      Cancer cells rely on lipogenesis in addition to exogenous lipid uptake, and fatty acid synthase (FASN) is aberrantly overexpressed in myeloid leukemia, yet its role in leukemogenesis is unclear. We show that FASN is essential for leukemogenesis. Its genetic ablation impairs leukemic cell growth, survival, and clonogenicity in vitro, and reduces disease burden in vivo, without significantly affecting normal hematopoiesis. We further identify a platensimycin derivative compound MS-C19 as a potent FASN inhibitor. MS-C19 suppresses growth and clonogenicity in clinical acute myeloid leukemia (AML) samples. Mechanistically, FASN inhibition or deficiency activates lysosomal and inflammatory gene programs, inducing lysosomal membrane permeabilization and associated cell death but not lysosome biogenesis. We further identify that GRN, a lysosomal and neuroinflammatory gene, is potently transcribed by TFEB upon FASN inhibition. GRN depletion reverses the anti-leukemic effects of FASN loss. Our findings establish FASN as a therapeutic target and support its pharmacological inhibition by MS-C19 for leukemia treatment.
    Keywords:  CP: cancer; fatty acid synthase; lysosome-dependent cell death; myeloid leukemia; platensimycin derivatives; progranulin
    DOI:  https://doi.org/10.1016/j.celrep.2026.117042
  13. Leuk Lymphoma. 2026 Mar 05. 1-8
      Myelofibrosis (MF) is a myeloproliferative neoplasm (MPN) characterized by progressive bone marrow fibrosis and extramedullary hematopoiesis. Patients with MF are at increased risk of heart failure (HF) and pulmonary hypertension (PH), which are associated with morbidity and mortality. Ruxolitinib is used in MF to palliate symptom. Whether treatment with ruxolitinib impacts risk of HF hospitalization or new PH in MF is unknown. This was a retrospective cohort of MF patients without prior HF. Outcomes were HF hospitalization or new PH and all-cause death. A total of 144 patients were included, 46 (31.9%) were on ruxolitinib, 40.3% were female and 78.5% were White race. Ruxolitinib treatment was associated with lower risk of HF hospitalization or new PH (adjusted subhazard ratio 0.27, 95% CI 0.10-0.73) but not all-cause death (adjusted HR 0.85, 95% CI 0.42-1.72). Prospective, randomized studies are needed to confirm the impact of ruxolitinib on cardiovascular outcomes among patients with MF.
    Keywords:  Neoplasia; myeloid leukemias and dysplasias; myeloproliferative disorders; pharmacotherapeutics
    DOI:  https://doi.org/10.1080/10428194.2026.2639642
  14. Blood. 2026 Mar 06. pii: blood.2025031402. [Epub ahead of print]
      Despite great progress in understanding the genomic basis of immature T-cell acute lymphoblastic leukemia/lymphoblastic lymphoma (T-ALL) and acute leukemias of ambiguous lineage (ALAL), there are still cases that lack defining genetic markers, complicating risk stratification and limiting targeted therapeutic options. Recent studies have shown that enhancer hijacking drives oncogene activation in approximately half of T-ALL cases, with the BCL11B enhancer frequently involved. Here, we describe a subtype of leukemia with a distinct gene expression signature, and immunophenotype characterized by positivity for immature (CD38), myeloid (CD13), T-lymphoid (cytoplasmic (c)CD3, CD7), and B-lymphoid markers (CD19, CD79a, CD10). This subtype is defined by the t(14;16)(q32;q24) translocation, which places the FOXF1 gene and its antisense long noncoding RNA gene FENDRR under the regulatory control of the BCL11B enhancer, leading to their ectopic transcriptional activation. Common concomitant genetic lesions are loss-of-function alterations of GATA3, CDKN2A/CDKN2B deletion and activating JAK/STAT and NOTCH1 pathway mutations. Patients were predominantly children and adolescents/young adults (AYA) and experienced poor treatment outcome. High-throughput drug screening of 176 compounds demonstrated efficacy of combined BCL2-family proteins and JAK/STAT signaling inhibitors. Additionally, the clinical use of tyrosine kinase inhibitors in some of these cases showed therapeutic efficacy. Collectively, these findings identify BCL11B-enhancer mediated deregulation of FOXF1/FENDRR as a hallmark of a subtype of high-risk lineage ambiguous leukemia that is potentially amenable to targeted therapeutic intervention.
    DOI:  https://doi.org/10.1182/blood.2025031402
  15. Blood Adv. 2026 Mar 05. pii: bloodadvances.2025018706. [Epub ahead of print]
      The phase 1 portion of SENTRY/XPORT-MF-034 (NCT04562389) evaluated the exportin 1 inhibitor selinexor (40 mg/60 mg once weekly) plus ruxolitinib in patients with JAK inhibitor-naïve myelofibrosis (n = 24). Primary endpoints were maximum tolerated selinexor dose, recommended clinical trial dose, and safety. No dose-limiting toxicities were reported. Common adverse events were nausea, fatigue, anemia, thrombocytopenia, constipation, vomiting, and headache. Nausea was transient, mainly grade 1, and managed by prophylactic antiemetics. Four deaths occurred, all unrelated to study treatment. Selinexor 60 mg in combination with ruxolitinib was identified as the recommended phase 3 dose based on safety, efficacy, and exposure-response analyses. Overall safety profiles and grades of clinically significant adverse events were similar and generally manageable regardless of selinexor dose. A greater proportion of patients achieved spleen volume reduction equal to or greater than 35% (SVR35) and total symptom score reduction equal to or greater than 50% (TSS50) at Week 24 in the 60-mg selinexor group (79% and 58%) compared with the 40-mg group (38% and 25%). Among evaluable patients who received suboptimal ruxolitinib ≤5 mg twice-daily in the selinexor 60-mg group, SVR35 was observed in 100% (6/6) of patients and TSS50 was observed in 75% (3/4) of patients.
    DOI:  https://doi.org/10.1182/bloodadvances.2025018706
  16. Bone Marrow Transplant. 2026 Mar 06.
      Higher-risk myelodysplastic syndrome (HR-MDS) is a heterogeneous group of hematopoietic malignancies primarily affecting the elderly, characterized by ineffective hematopoiesis, cytopenias, and a risk of transformation to acute myeloblastic leukemia. This review outlines the current landscape of HR-MDS management, focusing on risk stratification, treatment options, and challenges. The International Prognostic Scoring Systems (IPSS-R and IPSS-M) classify patients into risk categories, integrating cytogenetics and molecular data to guide therapy. Hypomethylating agents remain the standard of care for non-transplant-eligible patients, though their efficacy varies, with median overall survival ranging from 13-19 months. Promising novel agents include anti-apoptotic drugs (e.g., venetoclax), mutation-targeted drugs (e.g., TP53, IDH1/2), signal transduction inhibitors, inflammation pathway inhibitors and immune checkpoint inhibitors. Combinations of hypomethylating agents and these novel agents have shown promise in early trials as initial or salvage therapy but have failed to improve survival in phase III studies. Allogeneic hematopoietic stem cell transplantation is the only potentially curative option, yet its applicability is limited by patient age, comorbidities, and donor availability. Post-transplant relapse monitoring via chimerism and measurable residual disease is critical, with preemptive donor lymphocyte infusion recommended for relapse prevention. Future research should focus on mutation-driven therapies and inclusive trial designs to optimize HR-MDS management.
    DOI:  https://doi.org/10.1038/s41409-026-02821-4
  17. Cell Death Dis. 2026 Mar 03.
      Ionizing radiation and chemotherapy significantly impair hematopoietic stem and progenitor cell (HSPC) function, increasing the risk of bone marrow failure and secondary malignancies. Mesenchymal stromal cells (MSCs), critical regulators within the hematopoietic niche, maintain HSPC quiescence, self-renewal, survival, and differentiation. However, the specific pro-regenerative signaling pathways activated by MSCs in human HSPCs remain incompletely defined. Here, we show that bone marrow-derived MSCs effectively suppress irradiation-induced apoptosis and preserve the in vivo repopulation capacity of human HSPCs. Transcriptomic analysis of HSPCs revealed a pronounced upregulation of CREB target genes following MSC co-culture, consistent with increased activation of the cAMP/CREB signaling pathway. Mechanistically, MSC-secreted prostaglandin E2 (PGE2) emerged as a key mediator of cAMP induced response in HSPCs. Notably, the protective effect of Forskolin/IBMX persisted for up to 72 hours post-irradiation and significantly enhanced HSPC self-renewal. At the molecular level, we revealed reduced pro-apoptotic ASPP1 and PUMA expression, elevated p21 and stabilized anti-apoptotic MCL1 and BCL-XL proteins in human HSPCs treated with cAMP pathway agonists. Overall, our findings highlight the pivotal role of PGE2/cAMP/CREB signaling axis as a central mediator of MSC-mediated protection of human HSPCs under genotoxic stress and identify pharmacological cAMP activation as a promising strategy to protect human HSPCs against DNA damage-induced hematotoxicity.
    DOI:  https://doi.org/10.1038/s41419-026-08502-w
  18. Lancet Haematol. 2026 Mar;pii: S2352-3026(25)00361-8. [Epub ahead of print]13(3): e169-e180
       BACKGROUND: QuANTUM-First is a randomised phase 3 trial in individuals with newly diagnosed acute myeloid leukaemia (AML) that is FLT3 internal tandem duplication (ITD) positive, showing a survival advantage for quizartinib versus placebo plus standard induction and consolidation chemotherapy with or without transplantation, followed by single-agent maintenance therapy. We evaluated the impact of quizartinib on patient-reported outcomes and health-related quality of life using the European Organisation for Research and Treatment of Cancer 30-item Core Quality of Life Questionnaire.
    METHODS: In this global, multicentre, randomised, placebo-controlled, phase 3 trial, we recruited adults aged 18-75 years, with FLT3-ITD-positive newly diagnosed AML or AML secondary to myelodysplastic syndrome or myeloproliferative neoplasm, and with an Eastern Cooperative Oncology Group performance status of 0-2. Participants were randomly allocated (1:1) to quizartinib (40 mg/day) or placebo plus standard 7 + 3 induction chemotherapy, and then received standard consolidation chemotherapy with high-dose cytarabine plus quizartinib (40 mg/day) or placebo, allogeneic haematopoietic cell transplantation (allo-HCT), or both, followed by maintenance with single-agent quizartinib (30-60 mg/day) or placebo for up to 36 cycles. Randomisation was managed via an interactive web and voice response system. Patient-reported outcome endpoints, assessed using the European Organisation for Research and Treatment of Cancer 30-item Core Quality of Life Questionnaire, were exploratory and analyses were based on the patient-reported outcome intention-to-treat analysis set. Treatment effects on patient-reported outcomes were assessed using the mixed-effects model for repeated measures, time to sustained improvement, and time until definitive deterioration analyses. A minimal clinically important difference score of 10 or higher for each subscale was defined as clinically meaningful. This trial is registered with ClinicalTrials.gov, NCT02668653, and is completed.
    FINDINGS: Participants were enrolled between Sept 27, 2016, and Aug 14, 2019. Of 539 randomly allocated participants, 509 (278 [55%] female and 231 [45%] male) were included in the patient-reported outcome analysis set, 254 in the quizartinib group and 255 in the placebo group. The overall median follow-up was 39·2 months (IQR 31·9-45·8). Baseline patient-reported outcome scores were similar between groups. The change in global health status and quality of life score (GHS-QoL) from baseline was above the minimal clinically important difference from consolidation onwards in both groups. The treatment difference (quizartinib minus placebo) in change from baseline for GHS-QoL (by mixed-effects model for repeated measures) was -2·0 (95% CI -4·8 to 0·7, nominal p=0·15), indicating no substantial difference between groups, further confirmed by time to sustained improvement (subdistribution hazard ratio [SHR] 1·126 [95% CI 0·904 to 1·403], nominal p=0·28) and time until definitive deterioration (hazard ratio 0·81 [95% CI 0·51 to 1·28], nominal p=0·37) analyses. Longitudinal analyses of the functional and symptom subscales showed no substantially different patterns between groups. For the functional subscales, the SHR ranged from 0·940 to 1·148 (0·737-1·544, nominal p=0·36-0·90). For the symptom subscales, the SHR ranged from 0·965 to 1·407 (0·720-1·989, nominal p=0·28-0·99).
    INTERPRETATION: The results indicate that quizartinib plus standard chemotherapy prolongs overall survival without adversely affecting patient-reported outcomes and health-related quality of life, with no substantial differences between groups. Future research in real-world settings is warranted to assess the generalisability of these patient-reported outcome results.
    FUNDING: Daiichi Sankyo.
    DOI:  https://doi.org/10.1016/S2352-3026(25)00361-8
  19. Blood. 2026 03 02. pii: blood.2025031983. [Epub ahead of print]
      Chronic graft-versus-host disease (cGVHD) significantly contributes to late mortality after allogeneic stem cell transplantation, with bronchiolitis obliterans syndrome (BOS) being a particularly lethal and treatment-resistant complication despite available therapies. Bromodomain and Extra terminal (BET) proteins are epigenetic readers driving inflammatory transcriptional programs across multiple cell types. We hypothesized that BET inhibition would suppress inflammatory T and B cells while also decreasing macrophage polarization to a profibrotic phenotype, alleviating disease. In an established BOS cGVHD model, BET inhibition reduced germinal center formation and response through a reduction of the CXCL13:CXCR5 axis, inflammatory Tfh/GC B cells in the spleen along with a reduction in plasma cell infiltration within the lung. Mice with cGVHD had elevated pathogenic IgG1 and IgM, both in circulation and deposited on lung tissue, which was attenuated under BET inhibition. Single-cell RNA sequencing analysis revealed distinct cell states in the BOS lung vs. control. In cGVHD mice, GSEA analysis revealed upregulation of profibrotic Arginase1 and Tgfb1 expression in alveolar macrophages (AM) and interstitial macrophages (IM), which was significantly reduced with BET inhibition. Furthermore, BET inhibition targeted lung-infiltrating M2 macrophages, through selective depletion of CD206+FcgR+ IMs and AMs, ultimately resulting in reduced collagen deposition and improved lung function. Our findings reveal a previously unrecognized mechanistic axis of BET regulation during cGVHD fibrosis and highlight BET inhibition as a promising therapeutic strategy.
    DOI:  https://doi.org/10.1182/blood.2025031983
  20. Nat Cell Biol. 2026 Mar 06.
      How cancer cells couple metabolic stress sensing to orchestrate specific survival programmes is a key question. Here we show a long non-coding RNA (lncRNA)-guided epitranscriptomic mechanism orchestrating metabolic adaptation by controlling the stability of master stress regulator ATF4. Glucose or glutamine deprivation induces endoplasmic reticulum stress via reactive oxygen species-NRF2-dependent transcription of the lncRNA DAMER. Following its demethylation and nuclear retention by the m6A-eraser ALKBH5, DAMER acts as a scaffold, guiding ALKBH5 to demethylate and stabilize ATF4 mRNA through specific base-pairing. This provides an alternative post-transcriptional pathway for ATF4 upregulation, rewiring asparagine metabolism to promote cancer cell survival under stress. Furthermore, we identified the US FDA-approved drug elbasvir as a potent inhibitor of the DAMER-ALKBH5 interaction. Elbasvir dismantles this adaptive programme, targeting tumour asparagine dependency and exhibiting potent antitumour effects in preclinical models. Our findings reveal a paradigm for lncRNA-guided RNA demethylation that solves a target specificity enigma and offers a strategy targeting metabolic adaptation in cancer.
    DOI:  https://doi.org/10.1038/s41556-026-01905-z
  21. Br J Haematol. 2026 Mar 03.
    Consensus Meeting Attendees
      Genomic technologies including next-generation sequencing (NGS) and arrays for cytogenetic anomalies are now standard of care in England for the diagnostic evaluation of patients with suspected haematological malignancies. Challenges remain in the management of potential germline findings as a result of NGS panels and copy number variant analyses in haemato-oncology pathways. The first national consensus meeting in April 2022 organised by the UK Cancer Genetics Group (UKCGGG), in collaboration with the CanGene-CanVar research programme and the NHS England haemato-oncology working group led to published best practice recommendations on laboratory and clinical pathways where there was potential to identify germline predisposition to haematological malignancies. On 3 and 4 April 2025, a second national meeting was held to address further challenges in these pathways and review updates in the national landscape subsequent to the 2022 recommendations. The meeting specifically focussed on TP53, DDX41, myeloproliferative neoplasm driver genes, non-single nucleotide variation and identification of gene carriers for addition to the National Inherited Cancer Predisposition Register (NICPR) through the National Disease Registration Service (NDRS). Using the format of a pre-workshop survey followed by structured discussion and in-meeting polling, high-level consensus was achieved for UK best practice across these areas.
    Keywords:   DDX41 ; TP53 ; best practice guidelines; genetic testing; haematological malignancies; myeloproliferative neoplasms
    DOI:  https://doi.org/10.1111/bjh.70404